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Immunologically Defined Subclasses of Acute Lymphoblastic Leukaemia in Children: their Relationship to Presentation Features and Prognosis
Author(s) -
Greaves Melvyn F.,
Janossy George,
Peto Julian,
Kay Humphrey
Publication year - 1981
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1981.tb02704.x
Subject(s) - medicine , presentation (obstetrics) , lymphoblastic leukemia , immunology , pediatrics , leukemia , surgery
S ummary. Leukaemic cells from 542 patients under 21 years of age with a diagnosis of acute lymphoblastic leukaemia (ALL) were typed with immunological cell surface markers between June 1975 and December 1979; 379 of these patients entered into the trials up until December 1978 have been followed for more than 1 year. They were divided into four subgroups: common (c) ALL, T (thymic) ALL, ‘null’ (or ‘unclassified’) ALL and a rare lymphoma/leukaemia type B‐ALL. A T‐cell phenotype was found more frequently in boys and was usually but not invariably associated with a high white cell count at presentation. A mediastinal thymic mass was present in 53% of T‐ALL patients but was not observed in any unequivocal non‐T ALL. Clinical prognosis differed substantially between the three major phenotypic classes, remission induction rate and remission duration being lowest in T‐ALL, better in ‘null’ ALL, and highest in cALL ( P trend < 0·0001; P =0·0002 for comparison of cALL versus T‐ALL). There was a much higher incidence of CNS involvement in the T‐ALL group than in the cALL group or ‘null’ ALL group and although this was strongly correlated with WBC count it was also significantly associated with T‐ALL independent of WBC count. Overall in this series and also within the major cALL subclass there is a strong correlation between high WBC count and poor clinical response (remission induction and duration). When the three major immunological subclasses are adjusted for WBC count the prognostic correlation of antigenic phenotype is reduced and statistically insignificant. It is suggested that immunological (and enzymatic) phenotype of ALL subclasses may not be an independent correlate of prognosis but nevertheless is linked to other cell differentiation features, especially growth rate and sites of clonal expansion (e.g. marrow versus thymus), which critically influence the size of the clonogenic